Flares in pediatric systemic lupus erythematosus

Area of Science:

• Pediatric Rheumatology
• Systemic Lupus Erythematosus Research
• Clinical Disease Activity Monitoring

Background:

• Pediatric systemic lupus erythematosus (pSLE) is a chronic autoimmune disease requiring ongoing monitoring.
• Understanding disease flare rates and activity is crucial for effective management in children and adolescents.
• The Safety of Estrogens in Lupus Erythematosus: National Assessment Systemic Lupus Erythematosus Disease Activity Index (SELENA SLEDAI) is a key tool for assessing pSLE activity.

Purpose of the Study:

• To determine the flare rate in pediatric systemic lupus erythematosus (pSLE).
• To analyze the change in the SELENA SLEDAI score during disease flares in pSLE.
• To identify factors associated with a higher flare rate in pSLE.

Main Methods:

• Retrospective chart review of 62 patients diagnosed with pSLE (ages 5-20).
• Flare definition included initiation or dose increase of corticosteroids or addition of immunosuppressants.
• SELENA SLEDAI scores were recorded at pre-flare, flare, and post-flare visits; flare rate calculated per patient-year.

Main Results:

• The study included 62 patients, with 47 experiencing 112 flares.
• The pSLE flare rate was 0.46 flares per patient-year, with a median time to first flare of 14.3 months.
• Patients with cytopenia, pleuritis, pericarditis, or anti-Smith antibodies had significantly higher flare rates. The average SELENA SLEDAI score increased by 2 points during flares.

Conclusions:

• This study reports the first large flare rate for pSLE at 0.46 flares/patient-year.
• The pSLE flare rate is comparable to previous reports but lower than in adult lupus patients.
• A 2-point average increase in SELENA SLEDAI score indicates disease flare in pSLE.